We present two cases of persistent Mullerian duct syndrome (PMDS) with bilateral intra-abdominal testes and review the world's literature with special attention to diagnosis and surgical management. Two consecutive cases of PMDS with bilateral intra-abdominal testes from our institution are presented with detailed descriptions of the presentation, physical examination, laboratory profiles, surgical findings, and treatment undertaken. Follow-up at 1 year postoperatively is included. Bilateral orchiopexy was accomplished in both the cases. In one case this was possible after division of the persistent Mullerian structures in the midline to achieve testicular mobility. In a subsequent case, splitting of the Mullerian complex did not provide adequate mobilization and microvascular autotransplantation was performed with an excellent surgical outcome. Bilateral intra-abdominal testes in the setting of persistent Mullerian duct syndrome are a rare entity and controversy remains as to the ideal surgical treatment. Our two cases represent the first reported examples of open single-stage bilateral orchiopexy with division of the Mullerian complex and preservation of the vas deferens (1-year-old boy) and microvascular autotransplantation (5-year-old boy).